Abstract：
Invasive assessment of haemodynamics (ventricular, pulmonary) and testing of acute vasoreactivity in the catheterisation laboratory remain the gold standard for the diagnosis of pulmonary hypertension (PH) and pulmonary hypertensive vascular disease. However, these measurements and the interpretation thereof are challenging due to the heterogeneous aetiology of PH in childhood and potentially confounding factors in the catheterisation laboratory. Patients with pulmonary arterial hypertension (PAH) associated with congenital heart disease who have a cardiovascular shunt need to undergo a completely different catheterisation approach than those with idiopathic PAH lacking an anatomical cardiovascular defect. Diagnostic cardiac catheterisation of children with suspected PH usually includes right and left heart catheterisation, particularly for the initial assessment (ie, at the time of diagnosis), and should be performed in experienced centres only. Here, we present graded consensus recommendations for the invasive evaluation of children with PH including those with pulmonary hypertensive vascular disease and/or ventricular dysfunction. Based on the limited published studies and our own experience we suggest a structured catheterisation protocol and two separate definitions of positive acute vasoreactivity testing (AVT): (1) AVT to assess prognosis and indication for specific PH therapy, and (2) AVT to assess operability of PAH associated with congenital heart disease. The protocol and the latter definitions may help in the systematic assessment of these patients and the interpretation of the obtained data. Beyond an accurate diagnosis in the individual patient, such a structured approach may allow systematic decision making for the initiation of a specific treatment and may assist in estimating disease progression and individual prognosis.
摘要：
血流动力学的侵入性评估（心室，肺）和导管插入实验室中的急性血管反应性测试仍然是诊断肺动脉高压（PH）和肺动脉高压血管疾病的金标准。然而,由于儿童期PH的病因不均匀,以及导管插入实验室的潜在混杂因素,这些测量及其解释具有挑战性.患有先天性心脏病并伴有心血管分流的肺动脉高压（PAH）患者需要接受与缺乏解剖学心血管缺陷的特发性PAH患者完全不同的导管插入方法。疑似PH患儿的诊断性心导管插入术通常包括右心导管插入术和左心导管插入术。特别是对于初步评估(即，在诊断时)，只能在有经验的中心进行。这里,我们为PH患儿(包括肺动脉高压血管疾病和/或心室功能障碍患儿)的侵入性评估提供分级共识建议.根据有限的发表的研究和我们自己的经验，我们建议一个结构化的导管插入方案和两个单独的急性血管反应性测试（AVT）阳性定义：（1）AVT以评估特定PH治疗的预后和适应症。和（2）AVT评估与先天性心脏病相关的PAH的可操作性。协议和后者的定义可能有助于对这些患者的系统评估和对获得的数据的解释。除了对个体患者的准确诊断，这种结构化的方法可以为启动特定治疗提供系统决策,并且可以帮助估计疾病进展和个体预后.
